Open AccessMacrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report
Author(s) -
Rishikesh Kafle,
Anwesh Bhatta,
Sumit Gami,
Abhin Sapkota,
Dipesh Sharma,
Arabindra Yadav,
Vijaya Kumar Chikanbanjar
Publication year - 2021
Publication title -
journal of nepal medical association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.176
H-Index - 19
eISSN - 1815-672X
pISSN - 0028-2715
DOI - 10.31729/jnma.7019
Subject(s) - macrophage activation syndrome , medicine , hepatosplenomegaly , arthritis , hypofibrinogenemia , hypertriglyceridemia , juvenile , juvenile rheumatoid arthritis , immunology , pediatrics , disease , triglyceride , cholesterol , genetics , biology , fibrinogen
Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.